The mainstay of therapy is carbamazepine for paroxysmal kinesigenic dyskinesias and clonazepam for the nonkinesigenic dyskinesias. In symptomatic paroxysmal dyskinesias, the treatment of the underlying disease will provide best results. The ketogenic diet for patients with paroxysmal exertion-induced dyskinesias is a promising new therapeutic strategy and may not only prevent attacks but also lead to improvement of developmental delay in affected children.